Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023890 (cirrhosis)
 42,195 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Congenital dyserythropoietic anaemia Type II or HEMPAS (hereditary erythroblastic multinuclearity with positive acidified serum lysis test) is a rare genetic anaemia in humans, inherited in an autosomally recessive mode. Biochemical analyses of HEMPAS erythrocyte membranes suggested strongly that HEMPAS is caused by defective glycosylation of erythrocyte membrane glycoproteins. Most recently a HEMPAS case has been identified as being defective in the gene encoding Golgi alpha-mannosidase II by using cDNA probe of alpha-mannosidase II. At present, it is not clear whether HEMPAS is a genetically heterogenous collection of glycosylation deficiencies, as some HEMPAS cases showed a low level of N-acetylglucosaminyltransferase II. Abnormal glycosylation of serum glycoproteins and association of liver cirrhosis in HEMPAS patients indicate that HEMPAS disease is not restricted to erythroid cells. On the other hand, normal development of HEMPAS patients during embryonic stage strongly suggests the possibilities of fetal type isozyme in place of defective glycosylation enzyme.
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PMID:HEMPAS disease: genetic defect of glycosylation. 213 85

The patient, a 29-year-old female, was hospitalized because of clouding of consciousness, fever and right hemiplegia 4 days after the onset. On first examination she was found having fever in the 37 degree range, positive CRP, hepatomegaly, anemia and hepatic function impairment. Neurological examination revealed somnolence, conjugate deviation to the left and stiff neck. The muscular power measured about 3+ for the upper extremities and 0 for the lower extremities. Babinski sign was present on the right side. The spinal fluid showed an increase in cell counts, especially the neutrophil count. CT scans showed diffuse white-matter hypodensity in the left hemisphere. Soon after admission the patient fell into coma and died 6 days after admission. Autopsies led to a diagnosis of Hurst's encephalitis complicated by hepatoma with liver cirrhosis. It was reported that the immune complex was found in 25.9% of liver cirrhosis patients. From this fact it is suggested that Hurst's encephalitis might be elicited by some immunological mechanism.
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PMID:[A case of Hurst's encephalitis complicated by hepatoma]. 217 57

The authors report on the successful treatment of an unusual varix rupture causing intraabdominal hemorrhage in a patient who suffered from alcoholic hepatic cirrhosis. Simultaneously with the hypogastric pain, hypotension, growing circumference of the abdomen and increasing anaemia--without hematemesis or melena--referred to intraabdominal hemorrhage, which was confirmed by diagnostic paracentesis. Urgent exploratory laparotomy revealed the source of the bleeding and made the adequate surgical treatment possible. The authors are of the opinion that in case of a suspected intraabdominal hemorrhage the exploratory puncture is of diagnostic value and an operation performed without delay may ensure the improvement of the prognosis of this severe complication.
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PMID:[Hemorrhage in the abdominal cavity caused by ruptured varices]. 231 81

Alterations of the gallbladder wall is a well known sonographic sign of acute cholecystitis. But thickening of the gallbladder wall is also found in patients without intrinsic gallbladder disease. We present our experience on this regard in patients with cirrhosis, acute viral hepatitis, infectious mononucleosis, halothane hepatitis, fulminant hepatic failure, malaria due to plasmodium falciparum, heart failure, severe malnutrition due to gastric obstruction, septicemia, pyogenic hepatic abscess, amoebic hepatic abscess and in a 14 years old patient with fracture of the skull-acute anemia-shock. Most of these diseases affected the liver directly or indirectly. Knowledge of these alterations of the gallbladder wall in these circumstances are important in order to avoid a the erroneous diagnosis of acute cholecystitis.
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PMID:[Ultrasonographic changes in the gallbladder wall in non-gallbladder diseases]. 253 57

Biliary hemorrhage may occur in a variety of clinical settings, but spontaneous hemobilia has not been reported from a cirrhotic liver. We describe a case of major hepatic hemobilia in a patient with cirrhosis and no history of trauma. A 50-year-old woman had abdominal pain, melena, and profound anemia. An extensive workup did not show the site of bleeding but did show a mass in the gallbladder. Cholecystectomy was performed, and at operation the patient was found to have cirrhosis and portal hypertension. The gallbladder "mass" was simply an organized clot, and hemorrhage recurred postoperatively. On reoperation, bleeding from the ampulla of Vater was observed, confirming the diagnosis of hemobilia. She was treated with angiographic interruption of hepatic arterial flow, at which time bleeding ceased. Her total transfusion requirements included 46 units of blood. Through 16 months of follow-up the patient has had no recurrent bleeding and no evidence of encephalopathy. This case demonstrates that spontaneous hemobilia may indeed arise from a cirrhotic liver. Proximal interruption of arterial flow is usually not recommended for hemobilia, especially in the presence of portal hypertension and cirrhosis, but may be life-saving in selected patients.
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PMID:Atraumatic hemobilia arising from a cirrhotic liver. 266 59

Iron-chelating treatment is indicated in all children on prolonged transfusion therapy (i.e., chiefly patients with thalassemia and Blackfan-Diamond anemia). The purpose of iron-chelating treatment is to prevent the development of manifestations of iron overload including cardiac hemosiderosis and insulin-dependent diabetes mellitus (which are two potentially fatal complications), hepatic cirrhosis, hypoparathyroidism, hypothyroidism, and delayed puberty. Deferoxamine is the only effective iron-chelating agent and should be given in a daily dose of 40 mg/kg at initiation of the transfusion program. Administration is by subcutaneous infusions from 8 to 10 hours per day. The goal of iron-chelating treatment is to maintain serum ferritin levels between 500 and 1,000 ng/ml. This long-term treatment is a significant burden for patients and it can be hoped that non-toxic iron-chelating agents, active by mouth, will become available.
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PMID:[Iron chelation in children]. 268 51

Among the cattle admitted to the II. Medizinische Tierklinik of the University of Munich during the years 1982-86, 38 calves of up to 6 weeks of age had a dystrophy (n=5), pre-cirrhosis (n=21) or cirrhosis (n=12) of the liver. Similar hepatic lesions were found in three 1- to 7-day-old calves and in 1 foetus aborted in the 7th month of gestation which were submitted for necropsy. Of the 38 patients (74% male, 87% German Fleckvieh) 25 had been ill since the first seven days of life (mean age on admittance: x=14 days). These hepatopathies showed no significant preponderance in regard to breed, sex or season. The general condition of the calves was moderately to severely disturbed. Catarrhal enteritis resistant to treatment was the main clinical finding in 32 of the 38 patients. Nine calves developed central nervous symptoms (delayed reflexes, impaired coordination, convulsions, opisthotonus). Neurohistological examination of one of these calves revealed massive astrocytic oedema, shrinkage of individual nerve cells and formation of the so-called hepatic glia (hepatoencephalopathy). Even in the last days before death icterus of the sclera was noted in only 11 of the 38 patients; of these, nine also displayed icterus of the mucous membranes. The calves died between the third day to seventh week of life; mean age at death 18 days. Haematological examination: in 12 cases mild to marked anaemia (but without signs of haemolysis), marked granulocytosis with a shift to the left in 20 cases, sometimes additionally neutrocytes with nuclei resembling Pelger's nuclear anomaly (pseudo-Pelger), granulocytes with basophilic stippling as well as lymphoidocytes. The blood glucose levels were usually markedly reduced. 15 calves had a pronounced refractory hypoglycaemia with blood glucose levels under 1,66 mmol/l. Neither albumin concentration nor the specific serum globulin fractions showed significant differences when compared to an age matched control group.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Liver cirrhosis in young calves]. 276 85

In this study, background factors were determined in 108 surgical patients who showed depression in phagocytic and bacteriocidal activity with nitroblue tetrazolium reduction test. Control subjects consisted of 4 healthy males and 4 healthy females whose age ranged from 25 to 38 years. The background factors associated with the depressed neutrophil phagocytic and bacteriocidal activity in surgical patients were renal insufficiency, liver cirrhosis, hypoproteinemia, diabetes mellitus, long-term administration of steroids and immunosuppressants, obesity, anemia, aging and malignant tumors. These depression factors closely resembled those generally considered to be involved in increased susceptibility to infections.
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PMID:Depression factors of neutrophil bactericidal activity with nitroblue tetrazolium reduction test in surgical patients. 297 37

We examined hematopoietic disorders in 74 patients with various malignancies. The proportions of patients with anemia in the case of esophageal carcinoma, gastric carcinoma, colorectal carcinoma, hepatoma, and pancreatic carcinoma were 75%, 87.5%, 77%, 64% and 87.5%, respectively. Hypochromic microcytic anemia was mainly observed in showed patients with carcinoma of the gastro-intestinal tract which showed severe bleeding. The major proportion of patients with hepatoma and pancreatic carcinoma showed normochromic normocytic anemia. The WBC count was usually within the normal range except for patients with liver cirrhosis or generalized metastasis who showed decreased WBC counts. The mean lymphocyte count in the peripheral blood, which is thought to be correlated with the prognosis of cancer patients, was less than 1,500/microliter except for patients with gastric carcinoma, whose five-year survival rate was 28.6%. Monocytosis was mainly observed in patients with colorectal carcinoma and pancreatic carcinoma, accounting for 24.5% of the total cases. This finding may suggest some relationship between cancer and monocytes. Thrombocytosis was seen in patients with severe bleeding, but thrombocytopenia was seen in patients with liver cirrhosis. Most cancer patients showed normo-cellular marrow and normal M/E. We also examined the ferrokinetics and ferritin levels in cancer patients.
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PMID:[Hematological disorders in malignancy]. 301 96

Therapeutic action of blood components, preparations and substitutes was studied in liver cirrhosis patients with deep anemia, marked hemorrhagic complications, endogenous toxicosis, protein insufficiency and rheological disorders. A high efficacy of different media was shown in urgent therapy of liver cirrhosis patients. A choice of drugs was determined by the patients' initial status and specificities of purpose-oriented action of modern transfusion and infusion media.
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PMID:[Blood components, preparations and blood substitutes in the emergency therapy of patients with liver cirrhosis]. 336 54


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